ID 159 - Madelung’s disease. A case report

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• 作者：N. Ausí ; n Morales^a ; I. Lambarri San Martí ; n^a ; J. Barcena Llona^b ; A. Jauregui Barrutia^b ; A. Martí ; nez Zuluaga^a ; T. Vicario Elorduy^a ; I. Yurrebaso Santa Marí ; a^a
• 刊名：Clinical Neurophysiology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：127
• 期：3
• 页码：e62
• 全文大小：35 K

文摘

Madelung’s disease or multiple symmetric lipomatosis (MSL) is characterized by accumulation of unencapsulated fat, generally located symmetrically in the cervical region and back. Peripheral neuropathy is a common finding as well as the association with alcoholism, hepatopathy and glucose intolerance. Pathogenesis is unknown.

Methods

The authors present a case of 44 year old ex-alcoholic man with a 4 year history of multiple cervical lipomas being diagnosed of MSL. He developed symmetrical pain and weakness in lower limbs. Peripheral neuropathy was suspected based on distal amyotrophy and bilateral pes cavus deformities. Distal legs were thin with inverted-champagne bottle appearance. The study was based on radiological study and electrodiagnostic examination with electroneurography, needle electromyography and somatosensory evoked potentials.

Result

The electromioneurography study showed an intense sensorimotor polyneuropathy with a demyelinating and axonal component and signs of cervical polyradiculopathy. The cervical MRI showed cervical stenosis without significant cord compression.

Conclusion

MSL is a rare condition associated with alcoholism and high prevalence of peripheral neuropathy. Based on the neurophysiological examination and the clinical picture our patient presents a polyneuropathy caused by the toxic effect of the alcohol and with features of the hereditary motor and sensory neuropathy.

Key message

Madelung’s disease, peripheral neuropathy.